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Review
. 2022 Jul 28;11(15):4406.
doi: 10.3390/jcm11154406.

Structural Heart Alterations in Brugada Syndrome: Is it Really a Channelopathy? A Systematic Review

Antonio Oliva  1 , Simone Grassi  2 , Vilma Pinchi  2 , Francesca Cazzato  1 , Mónica Coll  3   4 , Mireia Alcalde  3 , Marta Vallverdú-Prats  3 , Alexandra Perez-Serra  3 , Estefanía Martínez-Barrios  5   6   7 , Sergi Cesar  5   6   7 , Anna Iglesias  3 , José Cruzalegui  5   6   7 , Clara Hernández  5 , Victoria Fiol  5   6   7 , Elena Arbelo  4   6   7 , Nuria Díez-Escuté  8 , Vincenzo Arena  9 , Josep Brugada  4   5   6   7   8 , Georgia Sarquella-Brugada  5   6   7   10 , Ramon Brugada  3   4   9   11 , Oscar Campuzano  3   4   10
Affiliations
Review

Structural Heart Alterations in Brugada Syndrome: Is it Really a Channelopathy? A Systematic Review

Antonio Oliva et al. J Clin Med. .

Abstract

Brugada syndrome (BrS) is classified as an inherited cardiac channelopathy attributed to dysfunctional ion channels and/or associated proteins in cardiomyocytes rather than to structural heart alterations. However, hearts of some BrS patients exhibit slight histologic abnormalities, suggesting that BrS could be a phenotypic variant of arrhythmogenic cardiomyopathy. We performed a systematic review of the literature following Preferred Reporting Items for Systematic Reviews and Meta-Analyses Statement (PRISMA) criteria. Our comprehensive analysis of structural findings did not reveal enough definitive evidence for reclassification of BrS as a cardiomyopathy. The collection and comprehensive analysis of new cases with a definitive BrS diagnosis are needed to clarify whether some of these structural features may have key roles in the pathophysiological pathways associated with malignant arrhythmogenic episodes.

Keywords: Brugada syndrome; endomyocardial biopsy; forensic pathology; histopathology; sudden cardiac death.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
PRISMA flow diagram followed in this review.
See this image and copyright information in PMC

References

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